Congenital partial absence of the pericardium in a young man with atypical chest pain

Abstract

Pericardial defects are infrequent congenital anomalies due to agenesis caused by premature atrophy of the common cardinal vein or Cuvier duct during the 5(th) or 6(th) week of embryonic life. These congenital defects are rare, typically observed as an incidental finding and usually remain asymptomatic. Nevertheless, the more widespread use of modern imaging techniques has contributed to an increase of its incidence in recent years. There is currently no consensus regarding therapeutic options, all of which are based on small retrospective studies that evaluate the risk of developing a life-threatening complication such as herniation and incarceration of the myocardium. We report on a 22-year-old male who presented with sudden onset of sharp chest pain and dyspnea. Computed tomography and cardiac magnetic resonance scan revealed a pericardial defect adjacent to the lateral free wall of the left atrium with associated herniation of the left atrial appendage. The patient was managed conservatively and had an uneventful clinical progress.